adults with congenital heart defects (ACHD) and pulmonary hypertension (PAH)/pulmonary vascular disease I27 Q24
Conditions
Interventions
Group 1: Adults with congenital heart defects and pulmonary hypertension or pulmonary vascular disease, for a time period of 12 months, with time points of 0, 4 weeks, 12 weeks, 6 months, 12 months wi
Sponsors
Deutsches Herzzentrum München, TUM Universitätsklinikum
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: • Age = 18 years • Presence of a congenital heart defect and pulmonary arterial hypertension • Patient at the ACHD center of the DHM • Ability to provide informed consent • Written informed consent
Exclusion criteria
Exclusion criteria: • Mental, cognitive, or language impairments that prevent the patient from participating in the study • Refusal to participate in the study
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The study involves the collection of comprehensive clinical and questionnaire-based data to systematically and precisely document the disease progression of pulmonary-arterial hypertension (PAH) in adults with congenital heart disease (ACHD). The following primary endpoints are evaluated: 1. Clinical Data Clinical Examination Findings: Including oxygen saturation, non-invasive blood pressure measurement, and non-invasive assessment of central aortic pressure. Echocardiography to evaluate systolic and diastolic heart function, assess right and left ventricular function, measure wall thickness, evaluate valve function, and estimate systolic pulmonary arterial pressure. Only if clinically indicated: Magnetic Resonance Imaging (MRI): To provide detailed visualization of pathoanatomy and precise quantification of functional parameters (e.g., determination of right and left ventricular volumes and masses, systolic and diastolic function), as well as myocardial perfusion to identify ischemia or fibrosis. Additionally, it includes imaging of the pulmonary arteries and their wall thickness and quantification of pulmonary blood flow. Invasive Pressure Measurement via Right and Left Heart Catheterization: To directly determine pulmonary arterial pressure and resistance values, as well as biventricular pressures and function. 2. Laboratory Values Extended Standard Laboratory Panel: Including electrolytes, kidney function parameters (creatinine, urea), liver function parameters, and inflammatory markers (e.g., CRP). Also included are metabolomic parameters (e.g., iron metabolism) and hemostaseological parameters. Analysis of biomarkers such as BNP, NT-proBNP, and additional biomarkers. 3. Patient-Reported Outcomes (PROs) Use of the clinically validated PAH-SYMPACT questionnaire to capture quality of life, subjective symptom perception, daily life impairments, and the impact of PAH medication adjustments. The questionnaire comprehensively covers physical symptoms, psych | — |
Secondary
| Measure | Time frame |
|---|---|
| Additional Research Questions: Identification of associated medical factors influencing the psychosocial state of ACHD patients with PAH: How does the psychological state vary concerning CHD-specific parameters? How does the psychological state of ACHD patients with PAH compare to other patient groups? How do patient-reported outcomes (i.e., subjective symptom perception and impact on daily life) in PAH differ from the clinical presentation of affected individuals? | — |
Countries
Germany
Contacts
Public ContactHarald Kaemmerer
Deutsches Herzzentrum München, TUM Universitätsklinikum
Outcome results
None listed